Gerstmann Syndrome

Synonyms of Gerstmann Syndrome

Developmental Gerstmann Syndrome

Gerstmann Tetrad

GS

General Discussion

Gerstmann syndrome is a rare neurological disorder that can occur as the result of a brain injury or as a developmental disorder. The syndrome is characterized by the loss or absence of four cognitive abilities- the loss of the ability to express thoughts in writing (agraphia, dysgraphia), to perform simple arithmetic problems (acalculia), to recognize or indicate one's own or another's fingers (finger agnosia), and to distinguish between the right and left sides of one's body. Additional cognitive defects may occur in some cases.

The disorder has not been found to run in families. In extremely rare cases, children who are bright and functioning intellectually at a high level may be affected by the disorder as well as those who suffer brain damage.

Gerstmann syndrome is different from Gerstmann-Sträussler-Scheinker syndrome, a rare genetic degenerative brain disorder.

Signs & Symptoms

Gerstmann syndrome is a rare disorder characterized by the loss of four specific neurological functions: Inability to write (dysgraphia or agraphia), the loss of the ability to do mathematics (acalculia), the inability to identify one’s own or another’s fingers (finger agnosia), and inability to make the distinction between the right and left side of the body. It is very rare for a person with learning disabilities to have all four of these neurologic dysfunctions. Only when all four symptoms appear together without mental retardation is the classic syndrome present.

When affected individuals have all four of the characteristic symptoms of Gerstmann syndrome without other cognitive defects, the condition may be referred to as “pure” Gerstmann syndrome. However affected individuals usually have other defects in addition to the classic four findings of Gerstmann syndrome. In addition, many individuals have only two or three of the four key findings in combination with other types of cognitive defects.

In such cases in addition to the four classical symptoms, affected individuals may also have difficulty expressing themselves through speech, and/or difficulty understanding another person’s speech (aphasia). They may experience difficulty in reading and spelling as well.

A few cases have been reported in children and called developmental Gerstmann syndrome. These cases usually become apparent when children begin school. Affected children may demonstrate poor handwriting, spelling and math skills (e.g., difficulty adding, subtracting, dividing and multiplying). Some children have difficulty reading or understanding written words (alexia) and difficulty copying or tracing simple objects (constructional apraxia).

Some researchers suggest that developmental Gerstmann syndrome is not a true, unique syndrome, but rather a group of symptoms caused by another, underlying disorder.

Causes

In adults, the syndrome can arise in adults as a result of impaired blood flow to the brain (cerebrovascular disease) such as a stroke or other damage to the brain. The parietal lobes (upper side lobes) of the brain are affected in Gerstmann syndrome. The parietal lobes are involved with sensation and perception as well as understanding sensory input.

In rare cases, traumatic brain injury or a brain tumor in the same region of the brain can cause the various symptoms associated with Gerstmann syndrome.

The cause of Gerstmann syndrome in children is often unknown. Although in some cases it may be linked to brain damage, children without brain damage can also be affected.

Affected Populations

Gerstmann syndrome affects males and females in equal numbers. The incidence of Gerstmann syndrome in the general population is unknown. The disorder was first described by Dr. Josef Gerstmann, a Viennese neurologist, in 1924.

Related Disorders

Symptoms of the following disorders can be similar to those of Gerstmann syndrome. Comparisons may be useful for a differential diagnosis:

Alzheimer disease is a progressive condition of the brain that affects memory, thought, and language. The degenerative changes of Alzheimer disease lead to patches or plaques in the brain and the entanglement of nerve fibers (neurofibrillary tangles). Memory loss and behavioral changes occur as a result of these changes in brain tissue. The characteristic findings associated with Gerstmann syndrome can occur due to Alzheimer disease. (For more information on this disorder, choose “Alzheimer’s” as your search term in the Rare Disease Database).

Additional disorders may cause brain dysfunction and could potentially cause the four characteristic findings associated with Gerstmann syndrome. These characteristic findings have also been seen in alcoholics, lupus, carbon monoxide poisoning, and lead poisoning.

Diagnosis

The presence in the adult of all four neurological symptoms suggests a diagnosis of Gerstmann syndrome, especially when other causes of these symptoms are ruled out. Among children, most cases are recognized at school age when the affected person has difficulty in math and writing. Affected children may also have problems in spelling, performing the basic four mathematical calculations, and distinguishing left from right. Also, they generally fail the finger identification test. Many, but not all such children will find it difficult to copy simple drawings (constructional apraxia).

Standard Therapies

Treatment

Treatment of Gerstmann syndrome in developmental cases will involve special education and related rehabilitation and counseling services. Neurological examination is necessary to tell the difference between the two causes of the condition. In adults, treatment of the underlying neurological condition is necessary. When brain injury or tumor is involved, surgery may be used to alleviate the condition. In some cases, the symptoms affecting adults with Gerstmann syndrome diminish over time.

Investigational Therapies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.

For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:

Toll free: (800) 411-1222

TTY: (866) 411-1010

Email: prpl@cc.nih.gov

For information about clinical trials sponsored by private sources, contact:

NORD's Rare Disease Database provides brief introductions for patients and their families to more than 1,200 rare diseases. This is not a comprehensive database since there are nearly 7,000 diseases considered rare in the U.S. We add new topics as we are able to do so, with the help of rare disease medical experts.

If you are seeking information about a rare disease that is not in this database, we would suggest contacting the Genetic and Rare Diseases Information Center (GARD) at the National Institutes of Health. NIH has the most complete database of rare diseases in the U.S.

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